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International Recommendations for Screening and Preventative Practices for Long-Term Survivors of Transplantation and Cellular Therapy: A 2023 Update.

As hematopoietic cell transplantation (HCT) and cellular therapy expand to new indications and international access improves, the number of HCTs performed annually continues to rise. Parallel improvements in HCT techniques and supportive care entails more patients surviving long term, creating further emphasis on survivorship needs. Survivors are at risk for developing late complications secondary to pretransplantation, peritransplantation, and post-transplantation exposures and other underlying risk factors. Guidelines for screening and preventive practices for HCT survivors were originally p

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… Fasciitis and polymyositis are cGVHD manifestations [ 206 , 208 , 209 ]. … Polymyositis usually presents with moderate to severe proximal muscle weakness and myalgia [ 202 ]. …

Positive Autoantibody Is Associated with Malignancies in Patients with Idiopathic Interstitial Pneumonias.

Various autoantibodies are associated with clinical outcomes in patients with idiopathic interstitial pneumonias (IIPs). We retrospectively analyzed the association between autoantibodies and malignancies in IIP patients. Comprehensive analyses of autoantibodies were performed using immunoprecipitation and enzyme-linked immunosorbent assays in 193 consecutive IIP patients. Cancer-related factors were analyzed using logistic regression analysis. In total, 22 of 193 patients (11.4%) with IIP had malignant disease. In univariate analysis, positivity for any autoantibody (odds ratio (OR), 3.1; 95%

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… Some autoantibodies, in particular, myositis-specific autoantibodies such as anti-aminoacyl tRNA-synthetase (ARS) and anti-melanoma differentiation-associated gene 5 (MDA-5) antibodies, were reported to be associated with the unique clinical features of polymyositis/dermatomyositis (PM/DM). … Comprehensive assessment of myositis-specific autoantibodies in polymyositis/dermatomyositis-associated interstitial lung disease Respir. Med. 2016 121 91 99 10.1016/j.rmed.2016.10.019 27888997 12. Yura H. Sakamoto N. Satoh M. Ishimoto H. Hanaka T. Ito C. Hasegawa T. Tanaka S. Miyamura T. …

Sporadic late onset nemaline myopathy with monoclonal gammopathy of undetermined significance: two cases with long term stability.

Monoclonal gammopathy of undetermined significance (MGUS) associated to sporadic late onset nemaline myopathy (SLONM) is a rare and severely disabling condition of quickly progressive limb girdle acquired myopathy. It is believed by some authors to be due to myotoxicity of light chain deposits. Two female patients were diagnosed with MGUS associated SLONM. In the first case, diagnosis was delayed by 6 years thus giving time for a severe generalized myopathy and cardiomyopathy to develop. A single anti-myeloma chemotherapy with lenalidomide markedly improved and stabilized the patient's conditi

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… of SLONM with MGUS (MGUS-SLONM) is often reported (e.g. 50 % of the 14 patients reported by Chahin et al. (2005), 15 and 61% of the 28 patients reported by Naddaf et al. (2019). 14 SLONM without MGUS may include cases of dermatomyositis, 16 HIV-associated myositis, 17 paraneoplastic myositis,18 or polymyositis … Typical nemaline bodies presenting in a patient with polymyositis. Med Electron Microsc 2002; 35: 167-72. 20. Mizuno Y, Mori-Yoshimura M, Okamoto T., et al. Two cases of sporadic late onset nemaline myopathy effectively treated with immunotherapy. …

Pharyngeal-Esophageal Malignancies with Dermatologic Paraneoplastic Syndrome.

Systemic changes often send signals to the skin, and certain neoplastic diseases of the internal organs can also trigger skin manifestations. In this article, the authors make clinical photography presentations of the patients seen at our clinic with dermatologic paraneoplastic syndromes within pharyngeal-esophageal malignancies, describe several paraneoplastic dermatoses, and also review high-quality scientific literature in order to be able to highlight the dermatological signs of pharyngoesophageal malignant tumors. The majority of our patients with paraneoplastic dermatoses, filtering for

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… Another retrospective study described 12 patients with internal malignancy among 64 patients with polymyositis and 28 patients with dermatomyositis. … Polymyositis and Dermatomyositis Associated with Malignancy: A 30-Year Retrospective Study: Polymyositis and Dermatomyositis with Malignancy. Int. J. Dermatol. 2002;41:729–734. doi: 10.1046/j.1365-4362.2002.01648.x. 20. …

Extraglandular Manifestations of Sjögren’s Syndrome (SS): Dermatologic, Arthritic, Endocrine, Pulmonary, Cardiovascular, Gastroenterology, Renal, Urology, and Gynecologic Manifestations

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… Differential diagnosis of extraglandular manifestations of SS • Most common area of diagnostic confusion between Sjögren’s, scleroderma (progressive systemic sclerosis, PSS), polymyositis, and SLE • Importance of ruling out other causes of morbidity including myocardial infarction, pulmonary emboli, … Gastrointestinal Manifestations Dysphagia is common in SS and is most often due to lack of saliva , but there have also been reports of esophageal dysmotility, similar to that seen in polymyositis or scleroderma [ 102 , 126 , 127 ]. …

The Kaleidoscope of Polyautoimmunity: An Odyssey of Diagnostic Dilemmas.

Diagnostic accuracy is of the utmost importance, both in the clinical setting and for research purposes. Mixed connective tissue disease (MCTD), rheumatoid arthritis (RA), Sjogren's syndrome (SS), and overlap syndrome (OS) frequently exhibit symptoms that mimic those of other conditions. Unfortunately, there is no singular definitive test for diagnosing these connective tissue diseases (CTDs), necessitating the reliance on expert opinions. Further complicating the matter, these diseases have overlapping clinical and serological features, and some individuals with one autoimmune disease may dev

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… Discussion Based on the clinical spectrum and antibodies associated with various phenotypes, systemic autoimmune diseases have been classified under six mutually exclusive diseases, which include systemic lupus erythematosus (SLE), systemic sclerosis (SSc), dermatomyositis (DM), polymyositis (PM), primary … Sharp and his colleagues in 1972, which was characterized by overlapping features of SLE, systemic sclerosis (SSc), and polymyositis/dermatomyositis (PM/DM), along with the existence of antibodies targeting the U1 small nuclear ribonucleoprotein autoantigen (U1 snRNP) [ 7 ]. …

Эффективность и безопасность иммуноадсорбции как спасательной терапии подстрекательских миопатий: доклад three - обзор дел и литературы.

Идиопатическая воспалительная миопатия (МИМ) резюмирует редкие, системные аутоиммунные условия, характеризующиеся главным образом провокационным повреждением скелетной мышцы. Несмотря на то, что мышечные мышцы повреждены в первую очередь, эти заболевания, связанные с IIM, связаны с другими органами, включая кожу, легкие, верхний желудочно-кишечный тракт, суставы и сердце. Несмотря на то, что многие пациенты адекватно реагируют на иммунодепрессивное лечение, некоторые пациенты развивают быстро прогрессивные и устойчивые к лечению жизнестойкие курсы. Для лечащего врача ИИМ является сложным делом

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… [Successful treatment of ‘steroid-immunosuppressant resistant’ polymyositis with immunoadsorption] . Rinsho Shinkeigaku 1991 ; 31 : 1208 – 1213 . 1813189 17 Sebastiani M Puccini R Manfredi A , et al . … Controlled trial of plasma exchange and leukapheresis in polymyositis and dermatomyositis . N Engl J Med 1992 ; 326 : 1380 – 1384 . 1472183 10.1056/NEJM199205213262102 22 Weeding E Tiniakou E . Therapeutic management of immune-mediated necrotizing myositis . …

Correlation Between Serum Tumor Marker Levels and Connective Tissue Disease-Related Interstitial Lung Disease.

OBJECTIVE: The main aims of this study were to explore the relationships between serum tumor markers and connective tissue disease-related interstitial lung disease (CTD-ILD) and to evaluate the clinical value of tumor markers for investigating interstitial lung disease (ILD) in patients with connective tissue disease (CTD). METHODS: The study included 235 patients with CTD (90 CTD without ILDs, 145 CTD-ILD). Clinical information and the levels of inflammatory and tumor markers, including carbohydrate antigen (CA) 19-9, CA125, carcinoembryonic antigen (CEA), CA153, and cytokeratin 19 fragment

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… Interstitial lung disease in polymyositis and dermatomyositis. Curr Rheumatol Rep . 2005;7(2):99–105. doi: 10.1007/s11926-005-0061-4 5. Mathai SC, Danoff SK. Management of interstitial lung disease associated with connective tissue disease. BMJ . 2016;352:h6819. doi: 10.1136/bmj.h6819 6. … CA15-3 and cancer associated serum antigen assays are alternatives to the KL-6 assay for measuring serum MUC-1 levels in patients with interstitial lung disease associated with polymyositis/dermatomyositis. J Rheumatol . 2002;29(9):2021–2022;author reply 2022. 20. …

Thrombosis in Neuromuscular Medicine: Current Evidence, Unmet Needs, and Future Directions.

Venous thromboembolism (VTE), comprising deep vein thrombosis and pulmonary embolism, is an important but under-recognised complication in neuromuscular diseases. In adults, emerging epidemiological data suggests increased VTE occurrence in conditions such as Amyotrophic Lateral Sclerosis, myotonic dystrophy, myasthenia gravis, inflammatory neuropathies, inflammatory myopathies, and POEMS syndrome. This heightened risk reflects not only disease-related immobility but also disorder-specific biological mechanisms, including inflammation, endothelial dysfunction and cardiomyopathy-related stasis.

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… A meta-analysis on 9045 patients with dermatomyositis (DM)/polymyositis (PM) showed that inflammatory myositis was associated with an increased risk of VTE, with a pooled odds ratio of 4.31 (95% CI, 2.55–7.29) [ 92 ]. … Cardiovascular events in adult polymyositis and dermatomyositis: A meta-analysis of observational studies. Rheumatology. 2022;61:2728–2739. doi: 10.1093/rheumatology/keab851. 18. Sayar Z., Weatherill A., Keddie S., Sive J., Lunn M.P., Thomas M., D’sa S. …

Determinants and Clinical Significance of Musculoskeletal Symptoms in Patients With Chronic Graft-Versus-Host Disease.

Musculoskeletal symptoms in chronic graft-versus-host disease (cGVHD) are rare manifestations contributing to disease burden. This study assesses the frequency of muscle cramps, joint and muscle aches, and muscle weakness in a cohort of patients severely affected by cGVHD. Three hundred thirty-four patients participated in the NCI natural history study of cGVHD (NCT00092235) from October 2004 to March 2017. Five-point Lee cGVHD Symptom Scale was dichotomized (less symptom bother-0, 1, 2; severe symptom bother-3, 4) and tested for associations with: Short Form 36 (SF36), 2-minute walk test, gri

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… Diagnostic features of musculoskeletal cGVHD are fasciitis, joint stiffness, and contractures, whereas distinctive features include myositis and polymyositis. … The NIH cGVHD organ scoring system records the presence of other musculoskeletal cGVHD-related features, including peripheral neuropathy and polymyositis, but these do not contribute to the total scoring of disease severity. …

Stem Cell Therapy in Neuroimmunological Diseases and Its Potential Neuroimmunological Complications.

Background: Since the 1990s, transplantations of hematopoietic and mesenchymal stem cells (HSCT and MSCT) and dendritic cell (DCT) have been investigated for the treatment of neurological autoimmune disorders (NADs). With the growing number of transplanted patients, awareness of neuroimmunolgical complications has increased. Therefore, an overview of SCT for the most common NADs and reports of secondary immunity after SCT is provided. Methods: For this narrative review, a literature search of the PubMed database was performed. A total of 86 articles reporting on different SCTs in NADs and 61 a

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… Interestingly, there were two reports of patients in which onset of myasthenia gravis and concomitant polymyositis was described [ 165 , 166 ]. … Autologous Bone Marrow Transplantation for Polymyositis Combined with Myasthenia Gravis and Aplastic Anemia: A Case Report. Case Rep. Neurol. 2018;10:108–111. doi: 10.1159/000488476. 125. …

Neurology

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The Production Processes and Biological Effects of Intravenous Immunoglobulin.

Immunoglobulin is a highly diverse autologous molecule able to influence immunity in different physiological and diseased situations. Its effect may be visible both in terms of development and function of B and T lymphocytes. Polyclonal immunoglobulin may be used as therapy in many diseases in different circumstances such as primary and secondary hypogammaglobulinemia, recurrent infections, polyneuropathies, cancer, after allogeneic transplantation in the presence of infections and/or GVHD. However, recent studies have broadened the possible uses of polyclonal immunoglobulin showing that it ca

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… Intravenous immunoglobulin therapy in adult patients with polymyositis/dermatomyositis: A systematic literature review Clin. Rheumatol. 2012 31 801 806 10.1007/s10067-012-1940-5 22274797 118. Dalakas M.C. Illa I. Dambrosia J.M. Soueidan S.A. Stein D.P. Otero C. Dinsmore S.T. McCrosky S. … Intravenous gamma globulin as first line therapy in polymyositis and dermatomyositis: an open study in 11 adult patients J. Rheumatol. 1994 21 1092 1097 7932419 122. Dalakas M.C. Sonies B. Dambrosia J. Sekul E. Cupler E. Sivakumar K. …

Autoimmune Disease Risk With GLP-1RA, DPP-4i, and SGLT2i Treatment in Patients With Diabetes.

OBJECTIVE: Dipeptidyl peptidase-4 inhibitors (DPP-4i), glucagon-like peptide-1 receptor agonists (GLP-1RA), and sodium-glucose cotransporter-2 inhibitors (SGLT2i) are widely used for type 2 diabetes, yet their comparative immunologic safety is uncertain. To address this gap, we evaluated autoimmune disease incidence among patients treated with these agents. METHODS: We conducted emulated target trials using electronic health record data from 152 health care organizations in the TriNetX network (2016-2023). Adults with type 2 diabetes initiating DPP-4i, GLP-1RA, or SGLT2i monotherapy and no pr

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… arthritis, psoriasis, psoriatic arthritis, multiple sclerosis, inflammatory bowel disease, antineutrophilic cytoplasmic antibody‐associated vasculitis, giant cell arteritis, dermatomyositis, systemic sclerosis, celiac disease, autoimmune thyroiditis, myasthenia gravis, pemphigus, bullous pemphigoid, polymyositis … Myasthenia gravis 63 51 1.11 (0.77–1.59) 0.784 52 39 0.98 (0.65–1.48) 0.906 56 43 1.10 (0.73–1.64) 0.757 Pemphigus 20 10 1.94 (0.88–4.24) 0.269 22 18 0.92 (0.47–1.70) 0.891 13 14 0.77 (0.39–1.65) 0.759 Bullous pemphigoid 110 46 1.78 (1.24–2.46) 0.041 94 34 2.07 (1.34–3.05) 0.005 44 29 1.35 (0.84–2.11) 0.481 Polymyositis …

Полиаутоимунитет и множественные аутоиммунные синдромы: забытая клиническая проблема.

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… To enhance clarity, the classification is summarized below: Table 1 Classification of multiple autoimmune syndrome (MAS) types and their associated autoimmune diseases Type Autoimmune diseases included Type 1 Myasthenia gravis, thymoma, polymyositis, giant cell myocarditis Type 2 Sjögren’s syndrome, …

Adaptation of the Charlson Comorbidity Index for Register-Based Research in Sweden.

PurposeComorbidity indices are often used to measure comorbidities in register-based research. We aimed to adapt the Charlson comorbidity index (CCI) to a Swedish setting.MethodsFour versions of the CCI were compared and evaluated by disease-specific experts.ResultsWe created a cohesive coding system for CCI to 1) harmonize the content between different international classification of disease codes (ICD-7,8,9,10), 2) delete incorrect codes, 3) enhance the distinction between mild, moderate or severe disease (and between diabetes with and without end-organ damage), 4) minimize duplication of co

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… arteritis), SLE, Takayashu arteritis) | 446 (Polyarteritis nodosa) 696,00 (psoriasis-artrit, psoriatic arthritis) 712,0–712,3 (Reumatoid artrit, rheumatoid arthritis and ankylotic spondylitis) 712,5 (Ankyloserande spondylit, ankylotic spondylitis) 716 (Dermatomyosit och polymyosit, dermatomyositis and polymyositis … 734,0 (Systemisk skleros, systemic sclerosis) 734,1 (SLE) 734,9 (Sjögrens syndrom, Sjögren syndrome) | 446 (Polyarteritis nodosa) 696A (psoriasis-artrit, psoriatic arthritis) 710A-E (SLE, sklerodermi, Sjögrens syndrom, dermatomyosit, polymyosit; SLE, scleroderma, sicca syndrome, dermatomyositis and polymyositis …

Interstitial Lung Disease and Pulmonary Fibrosis: A Practical Approach for General Medicine Physicians with Focus on the Medical History.

Interstitial lung disease (ILD) and pulmonary fibrosis comprise a wide array of inflammatory and fibrotic lung diseases which are often confusing to general medicine and pulmonary physicians alike. In addition to the myriad of clinical and radiologic nomenclature used in ILD, histopathologic descriptors may be particularly confusing, and are often extrapolated to radiologic imaging patterns which may further add to the confusion. We propose that rather than focusing on precise histologic findings, focus should be on identifying an accurate etiology of ILD through a comprehensive and detailed m

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… A retrospective review of clinical features and treatment outcomes in steroid-resistant interstitial lung disease from polymyositis/dermatomyositis. Respir Med. 2013;107:890–896. doi: 10.1016/j.rmed.2013.02.015. 122. … Comprehensive assessment of myositis-specific autoantibodies in polymyositis/dermatomyositis-associated interstitial lung disease. Respir. Med. 2016;121:91–99. doi: 10.1016/j.rmed.2016.10.019. 133. Harington J.S. …

Outcomes of Conservative Versus Surgical Treatment of Dropped Head Syndrome in a Single Institution: A Case Series and Review of the Literature.

Dropped head syndrome (DHS), also known as camptocephalia, is characterized by a mobile chin-on-chest deformity from hypotonia of the cervical extensor muscle or hypertonia of the anterior neck muscles. There remains a paucity of quality published literature on this topic, particularly its management. The objective of this study is to identify radiographic and clinical outcomes of surgery as compared to nonsurgical treatment, to summarize the literature, and to create a decision-making paradigm for managing patients with DHS. As such, we report outcomes of our retrospective chart review series

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… Muscular | Isolated neck extensor myopathy (INEM), primary inflammatory such as polymyositis, scleromyositis, isolated inflammatory axial myopathy, anti-GAD-associated inflammatory myopathy, primary non-inflammatory such as nemaline myopathy, myopathy with rimmed vacuoles, myofibrillar myopathy, necrotizing … Patients with underlying INEM without myositis, polymyositis, myositis, or other inflammatory processes should get steroids, with several authors describing a prednisone regimen lasting two to four months [ 19 , 26 , 30 - 33 ]. …

Benefit and danger from immunotherapy in myasthenia gravis.

In the last years, significant advances have improved the knowledge of myasthenia gravis (MG) immunopathogenesis and have enabled to realize new molecules with a selective action targeting compounds of the immunological system. This review discusses emerging treatments for MG, including complement inhibitors, neonatal Fc receptor targeting agents, and B cell interfering drugs, focusing on benefit and danger. In the second section of the review, several related adverse events of immunotherapy, including MGonset, are debated.

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… Otherwise, it was frequently associated with myositis (particularly necrotizing myopathy, but also polymyositis, dermatomyositis, and unspecific myopathy), and, less commonly, with myocarditis or a combination of myositis and myocarditis. … Giant cell polymyositis and myocarditis associated with myasthenia gravis and thymoma. Neuropathology. 2013;33:281–287. doi: 10.1111/j.1440-1789.2012.01345.x. 69. Garibaldi M, Fionda L, Vanoli F, et al. …